Cover article LOWELL HANDLER

First of two parts — much more than Moving beyond misconceptions to a diagnosis

By Samuel H. Zinner, MD Far more people have heard of Tourette syndrome than know what it actually looks and sounds like—or how it feels to the person who has it. That’s a major reason the diagnosis of this condition—the most severe disorder—is often missed. A change of perception begins with understanding the breadth and variability of symptoms and being aware of comorbidity.

ore than a century has passed since the ical figures (including the Roman Emperor Claudius, French neurologist Georges Gilles de la Wolfgang Amadeus Mozart, and 18th century English Tourette first described the condition literary scholar ) testify to the wide- that bears his name, a name familiar to spread awareness of TS across cultures and time, de- the lay public and health professionals spite (or perhaps because of) its perceived rarity. So it alike. Once considered so rare that neurologists might seems that the medical community trailed the un- expectM to witness the disorder perhaps once in their trained community by centuries in recognizing the professional lifetime, Tourette syndrome (TS) is, in syndrome. fact, common enough that virtually all pediatricians From the time of its initial medical description in will have several patients with this condition in their 1885 until the 1960s, medical experts viewed TS as a practice. Yet, despite its name recognition and the psychological disorder, and treatment was customarily number of people it affects, TS often goes undiag- directed toward psychotherapy. In the 1960s, the ob- nosed or misdiagnosed. Misconceptions about this tic served benefit of psychotropic medications in alleviat- disorder are customary, with the syndrome often per- ing tics reformed this perception, proving a clear neu- ceived as characterized by bizarre, fitful behaviors or rologic basis to the phenomenon. That discovery has comical outbursts of uncontrollable profanity. prompted a wide range of research-based interest in Colorful descriptions of motor and phonic tics in lit- understanding and classifying this varied disorder. erary characters (such as “Mr. Pancks” in Charles The tics of TS range widely in their severity, form, Dickens’s Little Dorrit and “Captain Hardcastle” in frequency, and intensity. Moreover, although tics are Roald Dahl’s autobiographical works1) and in histor- the hallmark of diagnosis, TS is associated with a range of comorbid conditions. When present, these conditions are usually more serious or disabling than DR. ZINNER is assistant professor of pediatrics and a developmental- the tics themselves. behavioral pediatrician at the University of Washington, Center on Human Development and Disability, Seattle. He is a member of the medical advisory The pediatrician can be most helpful to a child with board of the Tourette Syndrome Association, Inc. TS by recognizing signs and symptoms early in onset.

22 CONTEMPORARY PEDIATRICS Vol. 21, No. 8 KEY POINTS Dispelling misconceptions about Tourette syndrome and its diagnosis ▼Fallacy: Tourette syndrome (TS) is rare. ▼Fact: TS is estimated to occur in at least 1% of children. Based on this estimate and on the 2000 US population census, there are approximately 750,000 children with TS in the United States. ▼Fallacy: Shouting of obscenities is a defining characteristic of TS. ▼Fact: Relatively few patients with TS yell out obscenities. ▼Fallacy: Patients with TS are easily recognized by their tics. ▼Fact: Because tics can be suppressed, it is likely that you will observe few or no tics in patients with TS in the clinical setting. This does not exclude the diagnosis of TS. Moreover, most patients are affected only mildly and usually escape notice. ▼Fallacy: The presenting complaint in patients with TS is The diagnosis of TS (the focus of Part 1 of this two-part always tics. article) and its management (the focus of Part 2, which ▼Fact: Often, the presenting complaint will not be tics but begins on page 38) depend on recognizing the unique rather complications of a comorbid condition (e.g., attention clinical presentation of each child and directing an deficit hyperactivity disorder, obsessive-compulsive disorder) ongoing comprehensive and supportive care plan. ▼Fallacy: A diagnosis of TS syndrome is, for most people, catastrophic. The tics of Tourette: There is ▼Fact: Many people are relieved by the diagnosis and the no textbook presentation understanding that it brings, especially if the diagnosis has been Recent studies suggest that tics—mostly transient, missed for some time. Also, tics may be mild or present minimal interference. lasting less than one year—occur in 20% or more of schoolchildren.2 Tourette syndrome, in which the tics are chronic, may occur in 1% or more of mainstream cession, each bout lasting seconds or more. What are children, with a higher prevalence still among children known as complex tics involve more muscle groups, with special education needs.3,4 often have a more sustained duration or orchestrated The clinical manifestations of TS are extremely di- pattern, and may appear purposeful. Complex phonic verse, and no two patients present identically. Most tics more clearly involve linguistically meaningful have mild tics; the minority is severely affected. Tics are repetitive, sudden, involuntary or semivoluntary TABLE 1 productions. They are customarily classified as “simple” or “complex” and as “motor” (movement) or Diagnostic criteria “phonic” (sound-producing). Tics may appear to be for Tourette syndrome exaggerated fragments of ordinary motor or phonic 5 behaviors that occur out of context. ❑ Both multiple motor and one or more vocal tics have been TS is the most severe , marked by both present at some time during the illness, although not necessarily motor and phonic tics; Table 1 lists the diagnostic cri- concurrently. teria. Chronic tic disorders other than TS are charac- ❑ The tics occur many times a day (usually in bouts) nearly every terized by motor tics only or, less commonly, by phonic day or intermittently throughout a period of more than one year, tics only. Chronic motor tic disorders occur more and during this period there was never a tic-free period of more commonly than do TS, but follow a course similar to than three consecutive months. that of TS in terms of tic progression and comorbid ❑ The onset is before 18 years. affliction. The following discussion refers specifically ❑ The disturbance is not due to the direct physiological effects to TS. of a substance (e.g., stimulants) or a general medical condition So-called simple tics involve a single muscle or func- (e.g., Huntington’s disease or postviral ). tionally related group of muscles, and are generally Reprinted with permission from the Diagnostic and Statistical Manual of Mental brief, lasting less than a half second each. However, Disorders, Text Revision, Copyright 2000, American Psychiatric Association. simple tics may occur in repeated bouts of rapid suc-

August 2004 CONTEMPORARY PEDIATRICS 23 TOURETTE SYNDROME—TOWARD DIAGNOSIS

TABLE 2 Some simple and some complex tics

Simple Complex or middle adolescence. At onset, Motor tics Eye blinking Hand gestures motor tics usually are simple in Nose wrinkling Jumping nature, with fast, darting, and Jaw thrusting Touching purposeless eye blinking or neck Facial grimacing Pressing movements being the more com- mon ones. Over months or years, Shoulder shrugging Stomping motor tics may become increasingly Wrist snapping Facial contortions complex, involving more areas of Neck jerking Repeatedly smelling an object the body, generally progressing Limb jerking Squatting over time in a top-to-bottom and Abdominal tensing Deep knee bends central-to-peripheral direction. In Retracing steps addition, the movements may be- Twirling when walking come more elaborate or sustained Unusual posturing (e.g., a “frozen” position, and include combinations of such as holding the neck in a particular tensed movements, such as touching or position for one or more seconds) tapping, hopping, or holding un- (a sudden, tic-like vulgar, sexual, or usual postures, such as an out- obscene gesture) stretched neck, eyes deviated to (imitation of someone else’s movements) one side, or other dystonic postur- Phonic tics Sniffing Single words or phrases ing. Motor tics may also be self-in- Grunting Speech blocking (e.g., or other jurious to the skin (scratching Barking interruption to speech fluency) oneself, frequently licking the lips Throat clearing Meaningless changes in pitch, emphasis, or volume resulting in severe chapping, or of speech Blowing pulling out one’s hair), mucous (repeating one’s own sounds or words) Coughing membranes (biting the inside of (repeating the last-heard sound, word, the cheeks), spinal, muscular, or Snorting or phrase) orthopedic structures (violent Chirping (inappropriate expression of a socially head-jerking, bending, or muscle Clicking unacceptable word or phrase, such as an obscenity straining), or vision (poking at the or specific ethnic, racial, or religious slur) Sucking sounds eyeballs). Screaming Similarly, phonic tics usually are simple at onset, and are gener- ally meaningless noises, such as speech and language than do are not vocal tics. Though some- coughing, grunting, or barking. simple phonic tics. Table 2 lists what controversial, it is within ac- Over time, more complex phonic examples of simple and complex cepted clinical practice to include tics may appear, and can include motor and phonic tics. phonic tics like sniffing in the vocal palilalia: repetition of one’s own The words “phonic” and “vocal” tic category. words, such as “How are you? are often used interchangeably in Tics typically appear in early You? You? You?” sometimes with the TS literature when describing childhood (most often by 6 or 7 an exaggerated volume or pace to oropharyngeal and nasopharyngeal years), initially as simple motor the repeated words. Other com- noise-producing tics. Strictly tics, followed by phonic tics and plex phonic tics include echolalia speaking, a vocal tic is produced by more complex motor tics usually (repeating someone else’s words). the vocal cords; so, some phonic within the ensuing two years or so, Although widely associated with tics (such as sniffing) technically although sometimes not until early TS, coprolalia (the utterance of ob-

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FIGURE 1 Bouts of tics take on a fractal character over time Tics occur in bouts (A), Individual tics and these bouts occur A in bouts (B – E), which may explain the waxing and waning nature of tics (E). Seconds

B Bouts of tics

Hours

C Bouts-of-bouts of tics

Days D Bouts-of-bouts-of-bouts of tics

Weeks

E Bouts-of-bouts-of-bouts-of-bouts of tics Source: Leckman JF, Peterson BS, King RA, et al: Phenomenology of tics and natural history of tic disorders. Advances in – Tourette Syndrome 2001;85:1–14. Months Reprinted with permission. Time scenities or other socially inappro- tic (present or past) must be pre- ior lasting for seconds or minutes. priate remarks) occurs in only a sent for the condition to meet the The bouts themselves may occur in minority of patients. Because of its definition of TS. bouts over hours, such that there sensational potential, however, this Because a picture speaks a thou- are bouts of bouts. That pattern feature of TS is routinely exploited sand words, you can better famil- may continue for days, months, or in media portrayals. The common iarize yourself with the diversity of longer (Figure 1). misconception that coprolalia tics by viewing examples online at Many people with TS describe a must be present for a person to www.mdvu.org/multimedia/slides/ “premonitory urge” or sensory dis- have TS may delay or obscure the ts/default.htm. comfort in a muscle or muscle diagnosis. Tics generally occur daily, but group preceding the tic, which Note that the distinction be- typically wax and wane. This qual- builds over time.6 This urge may be tween motor and phonic tics may ity of increasing and decreasing fre- described as tension, pressure, a be artificial and misleading. quency of tics is evident both in tickle, an itch, or another sensory Technically, phonic tics are motor short-term and long-term segments experience. The urges are some- tics, just ones that produce sound, of time. As such, tics usually are times described less as a physical and classification of some tics as seen to have a fractal quality; in sensation than as a psychic phe- motor vs. phonic is not universally other words, tics may occur in nomenon, such as anxiety or anger. agreed on (e.g., a sniff or a tongue bouts over seconds or minutes sep- External stimuli, such as a noise, click). For now, at least, a phonic arated by periods of tic-free behav- a word, or an image, can pro-

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TABLE 3 Comorbid conditions that complicate not tic. However, voluntary sup- management of TS pression comes at the cost of grow- ing discomfort and distraction Attention deficit hyperactivity disorder caused by the unrelenting, unful- Obsessive-compulsive behaviors filled urge. Tic suppression is dis- or disorder ruptive and can be more debilitating and in progression or cessation Learning differences or disabilities than the tic itself. The suppression (Figure 2). Anxiety disorders may interfere with a child’s ability More than half of TS patients will Mood disorders to focus and can increase anxiety. have comorbid attention deficit hy- Sleep disorders Tic suppression usually results in a peractivity disorder (ADHD). The Executive dysfunctions rebound phenomenon at a later— presenting clinical signs of ADHD Self-injurious behaviors safer, as it were—time and place usually precede the onset of tics. Personality disorders (such as when the child returns Psychostimulant medication, such Oppositional defiant disorder home from school) during which as methylphenidate, is often used to the severity of the urges that had treat ADHD. As a result, there has been suppressed compels more been a longstanding misperception voke this urge in some patients. frequent and intense tics. that psychostimulant medication Performing the tic results in tem- Stress, anxiety, fatigue, and other causes or exacerbates tics (I will porary relief. Patients often de- high-level emotional excitement address this misperception in Part 2). scribe a sensation of needing to exacerbate tics. Certain situations Features of ADHD may include have the tic feel “just right” to may reduce, or invoke suppression hyperactivity and restlessness, diffi- achieve relief. A single tic may of, tics, such as settings or occa- culty with sustained focus and at- bring relief for some people (or, sions where it may be embarrassing tention, and distractibility. Loss of for a particular person, some of to tic—visits to the doctor’s office control is another com- the time), whereas for other peo- or speaking with friends,7 for ex- mon feature. Among TS patients, ple (or at other times) a rapid suc- ample. Tics usually do not occur some impulsive tendencies may, in cession of repeated tics or orches- during sleep, but are more likely to fact, be responses to premonitory trations of tics is required. In the happen at times and places of per- urges, a kind of a complex tic. case of self-injuring tics, the sense sonal relaxation, such as while Manifestations can include nonob- of relief may be possible only watching television at home. scene, complex, socially inappropri- once pain is experienced, al- ate behaviors,9 such as pointing out though such pain is very distressful Other conditions coexist an unpleasant body odor or a flaw to the person. more often than not in someone’s appearance. Children Children younger than 10 years About 90% of patients with TS with comorbid ADHD are more typically are unable to identify have one or more comorbid con- likely to suffer greater psychosocial these urges. For patients who do ditions8 (Table 3). These condi- and academic consequences,10 and describe awareness of an urge, tics tions are stand-alone diagnoses to have more disruptive or delin- can usually be suppressed to some that occur more often in patients quent behavior, than are those degree, particularly as the person with TS than in the general pop- without comorbid ADHD.11 grows older (i.e., as a child reaches ulation. Outward signs of co- Obsessive thoughts and compul- adolescence). To this end, tics may morbid conditions may not ap- sive behaviors occur in most chil- be said to be voluntary or semivol- pear as dramatic as tics and may dren with TS. In about one quarter untary in most patients with TS. therefore be overlooked. Each of of these children, the thoughts or Some patients report that, were these conditions can follow its behaviors are so severe that they there no sensory urge, they would own natural course, both in onset disrupt normal function or cause

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FIGURE 2 The exacerbating-remitting natural history of TS and its comorbid conditions symptoms of ADHD; tics that hinder efficient thinking or func- Exacerbation Possible remission tioning; strenuous energy spent Obsessive-compulsive behavior suppressing tics; and obsessions fo- Vocal tics (simple→complex) cusing on irrelevant details or com- pulsions to perform functionless Motor tics (rostral→caudal progression) and time-consuming behaviors, Attention-deficit-hyperactivity disorder such as counting to 10 every three sentences or rehearsing a song men- 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 tally before being able to answer Age (years) each test question. People with TS are prone to anx- ADHD, tics, and obsessive-compulsive behaviors frequently first present in a iety. Symptoms may include gener- predictable order and age range in patients with TS. Symptom severity typically alized anxiety, social or peaks in adolescence, often remitting in late adolescence. The natural course of symptoms beyond young adulthood is poorly defined. other simple phobias, panic at- tacks, and sleep difficulties. Mood Source: Jankovic J: Tourette’s Syndrome. N Engl J Med 2001;345:1184–1191. Reprinted with permission. disorders are common and may in- Copyright 2001 Massachusetts Medical Society. All rights reserved. clude depression or (chronic but low-grade depression), anxiety, and constitute obsessive- Intelligence is distributed normally and, possibly, . compulsive disorder (OCD). It may among children with TS, but learn- Episodic sudden and explosive rage be difficult to discriminate complex ing disorders prevent these chil- attacks occur in a minority of pa- tics from compulsive behaviors. dren from functioning at their in- tients with TS and may reflect an Some behaviors may appear more tellectual potential. One unifying underlying neurologic disturbance tic-like (such as evening things up, feature of many learning disorders or a severe effect of anxiety and touching something over and over and of ADHD is executive dysfunc- mood disturbances. Both anxiety until it feels “just right,” or creating tions. Executive functions describe and depression may also be out- symmetry in surrounding objects) goal-directed skills that allow us comes of stresses imposed by for which the unofficial term “com- to use intelligence efficiently the emotional interference and pultics” (compulsions that look (organizing, planning, decision stigmatizing of tics and comorbid like tics) has been coined. Other making, impulse control, social conditions. behaviors may appear more like skills awareness, prioritizing). Sleep-related problems include obsessions (such as a fear of conta- People with executive dysfunc- increased arousal, prolonged wake- mination leading to repetitive or tions may appear scatterbrained or fulness, and increased time to excessive hand washing) and typi- find themselves easily distracted sleep latency,12 and are more likely cally cause significant emotional despite attempts to concentrate. in patients who have comorbid distress. Still other behaviors may These people have difficulty with ADHD. appear to be a fusion of an impulsive behavior that is governed by rules There is growing evidence that behavior and a tic, such as the urge and do not easily learn to correct spectrum disorder may be a to touch a hot flame or stare at the their behavior from past mistakes. comorbid condition of TS, albeit an sun, for which the unofficial term Tics and other comorbid condi- infrequent one.13 “impulsion” has been coined. tions can impose learning diffi- One of the most regrettable out- Learning disorders, especially in culties above and beyond those comes in children with TS is poor math and written language, are also caused by learning disorders. self-esteem. These children are common comorbid conditions. Examples include the interfering often the target of mockery and

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What’s going on in the brain? Failed filters and overloaded circuits bullying by peers, or punishment f the brain were simple enough that we could understand it, we’d be and humiliation by intolerant fam- “Iso simple that we couldn’t.” So remarked Dr. Paul Greengard, ily members or teachers. Symptoms winner of the 2000 Nobel Prize in Medicine or Physiology. No surprise, of ADHD, social skills difficulties, then, that the neurologic basis of TS is unclear. That uncertainty is learning differences, and other reflected in inconsistent classifications given the condition: “Tourette comorbid aspects increase the syndrome” vs. “Tourette disorder.” A syndrome describes a set of signs likelihood of victimization. and symptoms without an identified cause, whereas a disorder suggests an understood cause and, often, a predictable course. What seems clear is Yet again, that the underlying mechanisms for pathology in TS and its comorbid genetics matters conditions are linked. Tourette syndrome occurs in all According to our current model for understanding the pathophysiology of TS, the problem lies in the area of neurotransmission. Normally, cultures and races. Although a fa- and other neurotransmitters regulate messages transmitted milial basis for TS is well estab- along an important brain circuit that includes the , prefrontal lished, its genetic basis remains un- and other cortex regions, and thalamus.1 One of the primary functions clear. What does seem clear is that of this circuit is to process and filter information that refines controlled the syndrome is not explained by a movement, thought, judgment, and the learning of behavior sequences. simple gene mutation. Instead, one In TS, messages that should be filtered out instead slip through, in part or more major “susceptibility 2 because of faulty dopamine regulation at critical points in the circuit. genes” are likely involved, probably The failure of appropriate filtering disinhibits thoughts, intellectual of an autosomal dominant inheri- organization, sensory experiences, and behaviors. tance pattern, some genes with mi- What bridges tics and comorbid phenomena is a so-called urge-relief nor effect, others more influential.14 cycle. The overlapping circuitry may result in sensory urges relieved by tics, cognitive obsessive urges relieved by compulsive behaviors, or a sense The interaction of these genes with of urgency relieved by an impulsive act (like blurting out an answer or possible environmental influences interrupting someone) or by another socially inappropriate behavior (such appears to determine just how the as making a sudden off-color statement or observation).3 Other related disorder is expressed. Influences manifestations that involve overlapping brain structures can result in may include male gender (TS is depression, executive dysfunctions (such as poor organizational skills or four times more common in males inefficient intellectual processes), or sudden loss of emotional control that than in females) and prenatal or may be expressed as rage. perinatal factors such as lower birth TS is a neurodevelopmental disorder so, as the brain develops, the form weight or nonspecific maternal of the disorder evolves. The consequence is an array of colorfully unique emotional stress. features that vary not only from person to person, but also within any When both parents have a tic affected individual over time. To capture the range of potential symptoms, it has been suggested that an alternative name for TS and related disorders disorder or a related comorbid dis- would be “developmental basal ganglia disorder,” implying a spectrum order such as OCD, the likelihood of disorders that may manifest as any or all of the behavioral phenotypes. of the child having TS increases Although it is essential that tics be present for a diagnosis, it is most markedly. While high, the precise reasonable to regard tics as one of several possible symptoms of this risk of developing TS (and/or co- filtering disorder. morbid conditions) among off- spring of parents with TS and/or REFERENCES 1. Swerdlow NR, Young AB: Neuropathology in Tourette syndrome: An update. Adv Neurol 2001;85:151 OCD is not established. An ongo- 2. Mink JW: Neurobiology of basal ganglia circuits in Tourette syndrome: Faulty inhibition of unwanted motor ing prospective study recently re- patterns? Adv Neurol 2001;85:113 3. Sheppard DM, Bradshaw JL, Purcell R, et al: Tourette’s and comorbid syndromes: Obsessive compulsive and ported that young children (most attention deficit hyperactivity disorder. A common etiology? Clin Psychol Rev 1999;19:531 from a large, multigenerational TS-linkage family) with two

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TABLE 4 Exploring the TS- and/or OCD-affected parents, difficulties, are likely to feel embar- differential observed for up to five years, were rassed or ashamed. Parents may three times as likely to develop tics feel a blend of personal failure, fear, diagnosis of TS than children with only one and anger. To reduce these percep- Wilson disease 15 TS- and/or OCD-affected parent. tions, a sensitive, empathic, and ac- Sydenham Another study showed 43% of cepting interviewing approach is Multiple sclerosis young children with a parent or essential when taking a history. It is Head injury sibling with TS developed some usually helpful to interview older Postviral encephalitis tic disorder during a four-year children and adolescents privately, 16 Direct effects of a substance (e.g., prospective study. as well as in the company of their neuroleptic agent) An important point is made with parents, to build a trusting alliance. (brief, simple, shocklike an adage borrowed from the field Interviewing parents alone can al- muscle contractions) of asthma: “All that tics is not low an opportunity to discuss the Spasms, including blepharospasm Tourette.” Children with mental impact that their child’s behaviors (often seen in pervasive retardation, autism, or another have on family or spousal relations. ) neurologically-based developmen- These approaches paint a picture of Compulsions tal disability may have tics for the broader, global experience of Transient tic disorder reasons unrelated to the genes Tourette syndrome. Chronic tic disorder that cause TS. In taking the history and per- forming the physical examination, PANDAS at play? be mindful not only of tics but of prehensive and include prenatal One controversial and intriguing clues to each of the comorbid con- and perinatal factors, develop- theory posits an immunologic trigger ditions and considerations in the mental history, neurologic history for tics and obsessive-compulsive differential diagnosis (Table 4). The (e.g., seizure, coordination, trauma, behaviors. PANDAS, or pediatric ability to suppress tics distinguish- sleep, atypical development), autoimmune neuropsychiatric dis- es the movements from other hy- and confirmation of adequate orders associated with streptococ- perkinetic movement disorders and hearing and vision. Ask about a cal infection, was first defined in is a useful diagnostic aid. Tics are family history of tics or comorbid 1998.17 The PANDAS theory pro- seldom uncertain when observed conditions. poses that antibodies produced directly. Exceptions include so- It is likely that several tics, past against group A -hemolytic strep- called compultics and impulsions, and present, will not be recognized, tococcal infection cross-react with as described. Another exception is either because they were never no- critical tissue in the brain and re- stereotypies, such as those seen in ticed or because they have been sult in abrupt onset or exacerbation children with pervasive develop- forgotten. Reviewing the list of of symptoms. Proponents of the . Such behaviors common tics (Table 2) can help the theory draw mechanistic similarity may include orchestrated behav- patient and family recall over- with Sydenham chorea, a manifes- ioral sequences with touching, tap- looked tics, and may be particularly tation of rheumatic fever. Beyond ping, spinning, or other actions. helpful in diagnosing TS when the scope of this review, PANDAS is Unlike tics, stereotypies tend to be there is any doubt about a history the subject of an excellent com- completely voluntary, intentional, of phonic tics. mentary by Singer and Loiselle.18 and self-soothing. A social history can include infor- For the most part, the physical mation about parenting and disci- Look to the history examination will be unrevealing. pline, family stressors, friendships, Children and adolescents with tics The money is in the history.19 The bullying, abuse, temperament, and alone, and those with comorbid medical history should be com- self-esteem. Psychosocial function

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sist, such ersatz reassurance may plant the mistaken belief that a nervous disposition is causing the tics, or that the child could stop the tics if only he or she really wanted to. can be facilitated through use of the neurologic examination should be More often than not, present- Pediatric Symptom Checklist (www. nonfocal in tic disorders, although ing complaints will not be tics dbpeds.org/pdf/psc.pdf, with scoring “soft neuromaturational” signs may but rather complications of a co- instructions at www.dbpeds.org/pdf/ be present, such as ambidexterity morbid condition. Complaints jellinek.pdf), available without cost. or bedwetting beyond the age of may include school failure, ab- Scholastic data, including aca- 5 years. dominal pain, or sleep problems demic performance and behavior Diagnostic laboratory, imaging, suggesting depression; social rating scales completed by teach- or electroencephalographic studies skills problems; hyperactivity; or ers and parents, are vital in the ini- are not indicated in the evaluation bedwetting. (Parents or patients tial assessment. Several of these for TS. On occasion, such studies may also misinterpret tics—com- scales are available free, such as may be indicated to rule out other plaining of “allergies” because of the Vanderbilt Assessment Scales specific diagnostic considerations eye blinking or sniffing tics, for in the National Initiative for Chil- when findings on the physical example.) In such cases, be on dren’s Healthcare Quality ADHD examination or history warrant the alert for tics. Consider having Toolkit (www.nichq.org/resources/ further investigation. parents provide videotaped samples toolkit). of the child having tics at home, Commonly, tics will not be ob- Diagnostic pitfalls or try to discreetly observe the served during the office visit. to sidestep child in the waiting area, where Remember that tics are often sup- Although the diagnostic criteria for the child may be less likely to pressible (especially in the practi- TS are straightforward, the diagno- suppress the tics. tioner’s office), and they wax and sis is often missed. Misconceptions Some families of children with wane. Some children will substi- about the syndrome explain much TS trek from physician to physi- tute more apparent tics with less ap- of this oversight. Most of the items cian in search of an answer, and parent ones in the clinical setting, listed in the differential diagnosis wait years before they are given a or will wait to release tics precisely are uncommon; tics are not. Also, correct diagnosis. The understand- when you are not looking at consider other tic disorders, in- ing that the diagnosis brings to pa- them. Others may disguise tics as cluding a chronic tic disorder tient and family, and the chance to seemingly purposeful movements, (identical to TS except that the begin to truly manage the condi- such as brushing hair out of the tics—past and present—are motor tion, is, for many who have or who face in concert with a head jerk- or phonic tics, not both) or a tran- live with someone who has TS, an ing tic, sometimes referred to as sient tic disorder (identical to a incredible relief. □ parakinesias. chronic tic disorder but the tics last References appear on page 36 A careful physical examination less than a year [and longer than Part 2 of this article begins on page 38. may reveal telltale consequences of four weeks]). tics. These stigmata may include In the absence of a careful his- The photos in Parts 1 and 2, which excoriated lesions on the skin from tory and exam, tics should never were taken for this article, show picking, ulcers on the buccal mu- simply be explained away by a 14-year-old girl who has Tourette cosa from biting, or circumoral telling parents that their child “will syndrome. chapping from lip-licking. The outgrow them.” When the tics per-

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